Uitkomsten darmrevalidatieprogramma voor kinderen met kortedarmsyndroom

Objective

To evaluate an intestinal rehabilitation program, introduced in 2001, for children who developed short bowel syndrome (SBS) after extensive intestinal resection.

Design

Retrospective cohort study.

Method

General information on disease status and outcome of intestinal rehabilitation plus demographic data on all SBS patients, treated in the programme between 2001 and 2009, were collected. The outcome measures were intestinal autonomy, intestinal and/or liver transplantation and survival.

Results

A total of 10 boys and 9 girls, born at a median gestational age of 36 weeks, were treated according to intestinal rehabilitation programme guidelines. Eight of them had been referred to our centre from elsewhere, 3 times as many as in 1991-2000. The causes of SBS were: intestinal atresia (n = 3), gastroschisis (n = 2), volvulus (n = 9), necrotising enterocolitis (n = 3) or strangulation (n = 2). Median remaining small-intestinal length was 35 cm (range: 10-70 cm). In 14 patients the ileocecal valve was still present and in all 19 patients at least 25% of the colon remained. The median follow-up period was 25 months (range: 50 days-9 years). Following total parenteral nutrition lasting a median 138 days (range: 41 days-11 years), 16 patients (84%) reached intestinal autonomy. Central venous catheter-related complications occurred in all of the patients; liver function disorders in 13 (68%) and failure to thrive in 5 (26%). One patient underwent an intestinal lengthening procedure. None of the patients needed an intestinal transplantation, but one patient underwent a liver transplantation for intestinal failure associated liver disease. Overall mortality was 11%: 2 patients died of abdominal sepsis.

Conclusion

The specialised intestinal rehabilitation programme led to intestinal autonomy in 84% of the patients with SBS.

Conflict of interest: none declared. Financial support: none declared.